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Idiopathic recurrent pericarditis

Disease definition

Idiopathic recurrent pericarditis is a rare autoinflammatory syndrome defined as recurrence of pericardial inflammation of unknown origin following the first episode of acute pericarditis and a symptom-free interval of 4-6 weeks or longer. Recurrent attacks of chest pain may be the sole presentation or the chest pain may be accompanied by pericardial friction rub, electrocardiographic or echocardiographic changes, pericardial effusion and increased C-reactive protein. Cardiac tamponade is a rare, life-threatening complication.

ORPHA:251307

Classification level: Disorder
  • Synonym(s):
    • Idiopathic relapsing pericarditis
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: I09.2
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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